Giant Cerebellar Cavernomas in Pediatric Patients: Systematic Review with Illustrative Case

INTRODUCTION: Giant cavernomas (GCMs) are angiographically occult vascular malformations of the central nervous system measuring at least 4 cm in diameter. These are rare lesions, especially in the cerebellum. As such, the clinical and radiologic features, surgical management, and outcomes of treatment for this condition are unknown. METHODS: We performed a systematic review of SCOPUS and PubMed databases for case reports and case series of histopathologically proven GCMs arising from the cerebellum in the pediatric population, and included a case managed at our center. Data on clinical presentation, imaging features, surgical treatment and outcomes of management were collected. RESULTS: A total of 10 cases were identified from the systematic review, including our case report. The patients' median age was 6 months, with a slight male sex predilection (1.5:1). The most common neurologic manifestations were bulging fontanelle in infants, and headache and focal cerebellar deficits in older children. Surgical excision was done in all cases, while 50% of the cases had CSF diversion surgery done prior to excision. In cases with reported outcome, complete neurologic recovery was achieved in seven while partial recovery was reported in one case. No deaths were reported at median follow-up of 12 months. CONCLUSION: Our case report and systematic review show that giant cerebellar cavernoma is a rare differential diagnosis for symptomatic hemorrhagic cerebellar masses in the pediatric age group. Mainstay of treatment is surgical excision, which can be associated with favorable outcome in most cases.